Background: Dermatofibrosarcoma protuberans (DFSP) is a rare type of skin cancer that arises in the deeper layers of the skin, most commonly on the trunk and limbs. The presentation of DFSP on the scalp is worth documentation due to its extremely rare occurrence in the literature. Case Description: We describe a case of a 41-year-old female presented with a lump on the back of the head with gradual enlargement. It was solitary with a size 19 × 12 × 10 cm with purulent discharge and intermittent pain. A computed tomography-scan showed an isodense mass at mid occipital without intracranial involvement. Tumor extirpation and the free flap were performed collaborated with plastic surgery. The patient was discharged unremarkably on the 7th postoperative day without any neurological deficits. Conclusion: Our case is distinct because, after 24 months without any adjuvant therapy and without any recurrence, the patient is still doing well. This is a very rare clinical entity.
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